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Patients with Behçet’s Syndrome (BS) may present with different neurological problems, which are either primary or secondary consequences of the systemic disease. The clinical and imaging evidence suggests that primary neurological involvement in BS may be divided into two major forms: The first form, seen in the majority of patients, is the parenchymal central nervous system (CNS) involvement. This...
Neurofibroma (NF) is one of the most commonly encountered benign peripheral nerve sheath tumors in the skin (Fig.a, b for line drawing). Because NF is composed of each element of the peripheral nerve, it most likely represents a hamartoma. Some authors, however, contend NF is a neoplasm as it is associated with mutation in the neurofibromatosis type 1 (NF1) gene. Furthermore, neurofibroma may undergo...
Malignant peripheral nerve sheath tumor (MPNST) of the skin is a rare superficial sarcoma, capable of locally destructive growth and systemic metastasis. By definition, MPNST arises from a preexisting peripheral nerve sheath tumor or a nerve de novo. In the former circumstance, nearly all arise from a neurofibroma with a high association with the plexiform variant. Rare deeper variants of MPNST with...
Glial and meningothelial proliferations of the skin are rare. In this chapter, nasal glioma (glial heterotopia), meningocele, and cutaneous meningioma are discussed. As a group, these lesions represent a spectrum of abnormalities related to abnormal closure of the neural tube or abnormal migration of neural crest cells during development; therefore, semantically some of them are not considered true...
Nonneoplastic neural proliferations are among the most commonly encountered peripheral nerve sheath tumors in the skin. This chapter describes the various hamartomas (neuromas) and reparative or reactive proliferations that develop following nerve injury. In general, hamartomatous lesions of the skin constitute proliferations of each of the normal elements of the peripheral nerve fiber including Schwann...
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